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      Long-Term Growth Hormone Therapy in Mitochondrial Cytopathy

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          Objective: To describe in a 5-year-old Caucasian male with mitochondrial cytopathy, a biochemical growth hormone (GH) deficiency associated with normal GH biological activity as evaluated by Nb<sub>2</sub> cell bioassay and normal serum IGF-I and IGFBP3 values increasing slightly after GH administration. Method: Serum GH concentrations were measured with a commercial immunofluorometric assay and with a biological assay, which uses the Nb<sub>2</sub> cell line. Serum IGF-I and IGFBP3 concentrations were measured with RIA. Results: The GH-supplementary therapy was initially effective in terms of growth gain, but no therapeutic benefit was observed over a long period of time. Conclusion: In patients suffering from mitochondrial cytopathy, short stature seems to be attributed more to a disease-related inadequate protein substrate than to the non-classical GH deficiency.

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          Diabetes mellitus associated with a pathogenic point mutation in mitochondrial DNA

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            Kearns-Sayre syndrome. A review of a multisystem disorder of children and young adults


              Author and article information

              Horm Res Paediatr
              Hormone Research in Paediatrics
              S. Karger AG
              September 2004
              13 September 2004
              : 62
              : 2
              : 103-106
              aDepartment of Pediatrics, University of Pavia, IRCCS San Matteo, and bDepartment of Child Neurology and Psychiatry ‘C. Mondino’ Foundation, University of Pavia, Pavia, Italy
              80451 Horm Res 2004;62:103–106
              © 2004 S. Karger AG, Basel

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              Page count
              Figures: 2, References: 17, Pages: 4
              Case Report


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